Saturday, July 19, 2014

Updated: TPL Shunt site better post-surgery. VP Shunt iffy? Discharged Fri (18th)

Sorry for the delay in writing an update; I am home (Fri) which if nothing else is nice! I hate writing these updates the most as I can imagine it must get tiring for those who read this to continually read about problems. I promise I would love to write all good updates for once to! =) To boot uncertainty makes me apprehensive (atleast in regard to the shunts) and it is a bit daunting (scary?) not having a plan.
I do atleast feel a bit better of the whole situation today than I did over the past few days. I do just want to feel better though!

Due to what I am more than certain is a valve issue and on-going, slowly worsening symptoms (VP Shunt) Dr.Bragg opted to do a shunt tap Fri morning which showed the valve is working but seemed was slow CSF flow (not entirely sure) when she was pulling off fluid. In the past this has been indicative of partial or intermittent shunt blockage; there have been numerous times we've just ended up revising that portion of the shunt (the valve).
She was able to remove with the shunt tap I think she said about 2cm (or 2cc's?) of CSF for which I can only best describe as it felt literally like a heavy cloud (cobwebs?) is partially lifted from my head. A very odd but not terrible feeling.
It's very clear the symptoms at night/morning especially had gradually gotten worse over the past few weeks. My symptoms after this shunt tap where virtually back to 'normal' by Sat morning, meaning I did not feel as good as I had headache wise after we did the shunt tan and took off some fluid.
I trust that there is a reason for not being certain what to do next but admittedly feeling like crap a good percent of the day and "just doing what I do at home" to get through it sucks (though I am happier to be here than to be in-pt). Atleast at home I control my day to day. It would be sooo amazing to just wake up and not have to feel aweful, literally feeling like your waiting as the day slowly gets better!

Even with some relief from the shunt tap (the first 24 hrs anyways) my vision is screwed up (fuzzy I guess, worse with reading) and head a day later feels like a lump of coal sitting on it just initially was not as bad as pre-tap! The symptoms do occur intermittently and worse at certain points of the day.
I think with the CSF that was pulled of with the tap she was planning to send off the CSF sample for testing though it is unlikely there would be an infection (no symptoms) and she I guess just wanted to be cautious, rule things out. I am ok with that, glad I guess she wanted to try and cover bases.
The bad thing with our relieving some pressure Fri morning is of course the headaches return and sometimes that is worse than not getting any relief till we figure out a actual plan! You can almost taste feeling better, longer with times like this where some pressure is relieved! Still I am glad even if it wasn't perfect relief to have felt a little better.
BR> We do know the TPL Shunt is working (a very good thing!!) based off flow during the TPL Shunt revision surgery on Monday. Thankfully the pinching/irritation symptoms from the TPL Shunt rubbing is also improved following surgery which is AWESOME! One of the incision areas from Monday's surgery surprisingly is still sore. This was done via laparoscopic (tiny) incisions but perhaps it's the location that causes the discomfort. ATLEAST the pinching/discomfort along the TPL Shunt is virtually (if not completely!) gone post-surgery!

As far as what is our plan, I believe Dr.Bragg when she says there are options but it doesn't make the time while waiting to figure out what the next step is any easier. Even Dr.Bragg seems to be struggling with what to do but better she takes her time to figure out a plan I guess. One possible thing she has circled back to is doing the cranial vault expansion surgery (where they go in a literally shave down some of the skull bone - if it sounds aweful to you believe me it sounds AWEFUL to me to!
The Peds Plastic Surgeon that helps Dr.Bragg w skull expansion surgeries is out till the 1st of August and I think it would likely be something we'd take some time to investigate (If it would work w the underlying MPS). I am more than leery of it!
In my case I think she said they would only (only!) need to shave down the skull bone and would not she believes need to reshape the actual skull. Does. Not. Sound. Awesome! I really don't know any other details about this but I think she is concerned we keep having to revise the shunts over and over and over again with limited success. It's to bad we couldn't figure out a way to replicate the TPL Shunt function (which we know works best out of the 2 shunts) in the VP Shunt!?
As far as recovery it seems if I am remembering Dr.Bragg right that is about 2 wks post-op in patient and they use plates and screws to hold the skull back together. Can you imagine? UGH!
I've talked to a couple drs in the MPS community about the potential skull bone thinning and both feel this may not work and little precedent (if any) for it's use in MPS. The one dr must have quickly done some research to see if she could find any published data on it's use and didn't have a lot of success.
The other dr who has a ton of experience (dozen of years) with MPS I pts brought back up the potential to look in to IT Aldurazyme via the shunt reservoir which is something I wish we could work out.
I guess i'll ask Dr.Bragg if it might be something we could look in to again and would she be willing to talk to Dr.Dickson (They had talked one other time)? I know there is 1 other MPS pt (not a shunt pt though) whose local dr was able to work w Dr.Dickson to get the IT (Aldurazyme) approved for out of study use.
Dr.Dickson has said she would be willing to try and help Dr.Bragg get it approved at UW if she wanted/was interested. I feel like we should try to look in to everything.. The idea behind IT in to a shunt is that they temporarily re-program the shunts to a 'virtual off' setting and inject the aldurazyme via a reservoir in-line w the shunt catheter.
Dr.Kakkis feels there would have to be a one way valve (not sure if the Codman Hakim is a one way valve). The thought is that Aldurazyme given directly in to a shunt reservoir may help clear our GAG storage shed from the ependymal
Dr.Kakkis also wondered if some type of reservoir could be used to flush the shunt catheter out with forced back pressure every once in awhile.. I am curious about this and something else to fwd to Dr.Bragg. I do know the catheters clogging is not unique to MPS pts but am unsure if it is more common with our disorder or a different unique? It seems it might not be but again feel like we have to look at everything..
Something I have wondered but didn't think of till after I talked to Dr.Bragg is I wonder can Hydrocephalus pts who do well draining a lot of fluid ever get shunted without an actual valve in place (valves control flow) on their shunt? Something to keep in mind to ask.. I would imagine a valve is needed but is worth asking.

I know as far as ideas she was going to talk to her Codman Rep about a siphon guard valve (controls amount of flow when person stands up) but she (and I for that matter) aren't sure that is a good option as I do better the more CSF I can drain.
I am doubtful she would decide to go that route and as much as I want to feel better I guess I am ok her taking her time trying to figure out a better option.

I guess I need to do better, trust whatever she decides and take these next few wks a day at a time.. I will do what I do well and that is enjoy the moments that are good while just living with the times that are not.
These periods in-between feeling good are definitely when my faith and trust are tested!

I'll update again when there is something to update,
Thanks for stopping by,
Erica
Ps Sorry I realize this update is fairly scattered and not all that well written!

Tuesday, July 15, 2014

TPL Shunt revision (#30-s/t), shorten pleural catheter

Although not bad news at all I really am forcing myself to update this, for whatever reason today I have little energy and typing this update out feels daunting!
I originally wrote out notes yesterday but then must have accidentally thrown that sheet out so am trying to remember details by memory on about 2 hrs of sleep (an hour-ish early this morning and an hour-ish around 12-1 or so. Neredless to say right about not I am feeling INCREDIBLY grouchy and irriated and just want peace with quiet with no one coming in here!
(One of the Pastors from the Hospital stopped by and honestly I asked him if he could come back in a little while, I just needed to be left alone to finish this and something else I was doing + am not really feeling like making small talk. Sometimes I just get soooo tired that I'd just like to be left alone!

Surgery which was scheduled at 1pm yesterday (arrival for labs at 9, check-in to 1st day surgery at 11) started about 1:30 (Dr.Bragg was in clinic and when she came by said she'd had an un-scheduled patient show up, not a big deal).
Really the Head Anesthesiologist and 2 Resident Anesthesiologists working w/her on my case had been standing around just chatting with my Dad and I while we waited.
I liked the whole team and could tell the head Anesthesiologist had done her homework on my past history + was well versed in the cardiac issues and what she would do differently. Her big concerns where to not cause fluid/cardiac overload and keep surgery (which was very short, intubation took much longer than the actual surgery) smooth.

Overall they did the pre-surgery cortisol replacement (there was discussion by the Anesthesia Team if it was really needed given the short length of the actual surgery but per what my Neuro-Endocrine dr has stressed to me numerous times the cortisol is as much for the induction/anesthetic drugs as it is for surgery. Cortisol deficiency with any stress of the body causes stress to the body systems and is why the cortisol has to be given 30-60mins prior to any anesthetic meds are given when possible.
It worked out fine as the Head dr said to me, along the lines of "You've been around the block w these surgeries and you know what your dr has told you regarding your case".

Surgery itself literally took about 15-20mins and if I remember correctly when they where hooking everything up, adding the anesthetic/induction meds it was about 2 hrs after this that I woke up in recovery. I am not exactly sure on any of the total time length though and doesn't much matter given surgery went good!
The Peds General Surgeon w/Dr.Bragg there basically just for "moral support for the Surgeons" (her words, lol) =) just used an endoscopic approach w 2 small incisions in order to view and to move their instruments.
They pulled out the pleural space (tip) of the TPL Shunt catheter, cut off about 1.5-2" and replaced it back in to the lung, which I believe they deflated for this surgery (a big concern the Anesthesiologist had given my airway issues and instruments she had to use).
Apparently per the Team they could visualize where the catheter was in fact sitting up against the chest wall and could see were it was causing irritation, which in turn explains my symptoms of irritation most especially when it seemed like the TPL Shunt would be trying to drain.
With the re-positioning of the catheter Dr.Bragg said it is short enough now where it should not touch any areas in the pleural space and as an added benefit we should get less resistance from the catheter which equals (we hope) more CSF flow through the catheter. That would be a good thing as we know I like to drain a lot and feel better the lower pressure is!
Thankfully the TPL catheter pinching/poking feeling has been pretty non-existent today and any discomfort there is either incisional or b/c Dr.Bragg said even with their draining excess fluid out of the pleural space during surgery there was still some that would absorb (they hope) on it's own.
Other than this positional catheter issue we've definitely had much better luck w the TPL Shunt than we have with VP Shunt functioning fully or well.

Even though the VP works less than optimally and I do have headaches/nausea/some blurred vision (intermittent) it as I've said before is still wayyyy better than when I only had 1 shunt and was endless problems.
I am sure it is at least somewhat improved now b/c the TPL Shunt takes some pressure off of the VP Shunt and they almost seem to balance each other with the TPL Shunt seeming to work more effectively and better, longer.

I feel like if we could figure out what causes the TPL Shunt to work more effectively than the VP Shunt and we could figure out a way to translate that effect to the VP Shunt we'd honestly be so much closer or perhaps as close to perfect as you could get with pressure symptoms.
Our biggest issue is Dr.Bragg is just unsure how to better improve the efficacy of the VP Shunt draining especially when I am asleep. I do sleep semi-propped up on my couch, always starting out sitting up but I think due to positioning/joints/neck I end up only partially upright.
I would LOVE it if we could figure out how to better make the VP Shunt work but hopefully we could keep brainstorming and figure something out in time? In the mean time you just do the best you can, someday's better than others with symptoms.

I will say as 'minor' as this surgery was ie very short and just 2 smaller incisions on my side and back even these smaller surgeries still kicks my butt both discomfort/pain wise and overall! =/
We are in the process of slowly weaning the PCA pump (patient controlled analgesic, I control the short acting pain med dosing based around how often Neurosurgery has it set that I can deliver med.) I am sure the difficulty with surgery recoveries in the first few days pain wise is due to so much scar tissue and so much GAG storage (from the MPS storage) which causes much tougher tissue.
Thankfully Dr.Bragg has a decent system in place for managing post-op pain w the PCA (self administered) pain pump. And she realizes for whatever reason my recoveries are just not a typical (whatever that is!) shunt patients recoveries.

I do know given we had turned up the VP Shunt setting by a notch a few wks ago it's possible if need be Dr.Bragg might also let us re-adjust that setting back down if H/A's don't improve.

Friday once I knew surgery would go ahead I called the Neurosurgery floor Pharmacists and they in-turn ordered the Aldurazyme to be given while here.
My Genzyme Insur. Nurse Case Man. was also watching for the order to make sure it go shipped out on Fri from their end.
This will likely be given on Weds morning now to give us time to fully wean off the PCA pump.
We'll also use the same ramp up rate this time that Stan (Nurse here) calculated last time. This is different than how it is ramped up at CHW, my normal clinic but something is done differently here maybe with how the drug is mixed as we otherwise where running the infusion at double the needed rate, giving it to quickly.
We'll also restart the Coumadin dosing tonight I believe and once released i'll go back to monitoring this via my Cardiology Team at CHW.

Due to my being in-patient the WI Team from Genzyme came up here to visit this morning which was actually really nice!
I'd only met Karen (Patient Care Liaison) before as she lives in the Minneapolis area and we frequently have dinner while I am there from time to time and see each other here and there at meetings.
Amy, my Insur. Case Man. at Genzyme (located Boston) as well as Carol, who is the WI Drug Rep for Genzyme (lives in Madison actually, a little bit newer to the team) where with Karen also and we had many good laughs, funny stories to share. We're all cat lovers so that was like an instant bond between Carol and I especially!
They all really liked the MPS-Aldurazyme bracelet (using the caps from the drug) bracelet I have/made and so my next project is to make one of each of these for them. I told them it would take atleast a few wks but I think it's just cool they liked the bracelet that much! =)
It's amazing the people in the Med world who really like this idea and it is neat b/c 1. they are intimately involved in MPS I pts lives everyday and 2. their company bottles the drug.
Jessi, my Boston Marathon runner also has one of these and had sent a pic of herself wearing it after she got it in the mail. =)

Going to sign off for now but if I've forgotten anything i'll re-update this and otherwise will update sometime again soon.
Hopefully I get out tomorrow or any day now; even if we still are dealing with headaches it's just nicer to be at home at a certain point!
Here are a couple pics from today with Amy, Karen and Carol this morning.

Thanks for stopping by,
Erica